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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 100-103

Oropharyngeal recovery in a patient with the pharyngeal cervical brachial variant of Guillain–Barré syndrome


Department of Rehabilitation Medicine, Tan Tock Seng Hospital, Singapore

Date of Submission10-Jan-2019
Date of Acceptance03-Mar-2019
Date of Web Publication29-Aug-2019

Correspondence Address:
Dr. Matthew Rong Jie Tay
Tan Tock Seng Rehabilitation Centre, 17 Ang Mo Kio Avenue 9, 569776
Singapore
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jisprm.jisprm_46_19

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  Abstract 


Patients with the rare pharyngeal cervical brachial variant of Guillain–Barré Syndrome present with a sudden and progressive onset in weakness of the pharyngeal, cervical, and brachial muscles, but usually make a rapid recovery. However, in a patient with delayed oropharyngeal recovery, early identification of poor clinical and electrophysiological prognostic factors can guide rehabilitation planning to optimize functional outcomes.

Keywords: Electrodiagnosis, Guillain–Barré syndrome, oropharynx, prognosis


How to cite this article:
Tay MR, Tham SL. Oropharyngeal recovery in a patient with the pharyngeal cervical brachial variant of Guillain–Barré syndrome. J Int Soc Phys Rehabil Med 2019;2:100-3

How to cite this URL:
Tay MR, Tham SL. Oropharyngeal recovery in a patient with the pharyngeal cervical brachial variant of Guillain–Barré syndrome. J Int Soc Phys Rehabil Med [serial online] 2019 [cited 2019 Oct 20];2:100-3. Available from: http://www.jisprm.org/text.asp?2019/2/2/100/263553




  Introduction Top


Guillain–Barré syndrome (GBS) is an acute monophasic immunomediated polyradiculoneuropathy which can present with unusual variants, and manifestations include generalized or selective weakness, areflexia, variable sensory loss, cranial nerve involvement, and dysautonomia. Although most patients recover rapidly, a minority experience severe disease and poor functional outcomes. Hence, goal-focused, time-specific, and function-based rehabilitation is often required to minimize disability after acute treatment. For example, patients with the rare pharyngeal cervical brachial (PCB) variant of GBS usually recover within weeks. Here, we report a patient with poor clinical and electrophysiological prognostic factors, resulting in delayed oropharyngeal recovery over 4 months which, to the best of our knowledge, is the longest in published literature. This emphasizes the need to identify prognostic markers early so that individualized multidisciplinary rehabilitation can be implemented expediently for such patients to maximize functional outcomes.


  Case Report Top


A 59-year-old male with no significant past medical history developed sudden onset of facial drooping, dysarthria, and dysphagia shortly after an upper respiratory tract infection. On admission, the patient was afebrile, alert, and oriented without any signs of meningeal irritation. He had bilateral lower motor neuron cranial nerve 7 palsies, weak tongue, weak palatal movements, and marked dysarthria. He was noted to have left shoulder abduction weakness and left upper limb areflexia. There was no fatigability, and sensory examination was normal. There was no gait ataxia. He developed rapidly progressive dysphagia over 1 day requiring intubation due to an inability to clear oral secretions. A nasogastric tube was inserted for dysphagia, and an indwelling urinary catheter was required due to urinary retention. An initial electrophysiological study did not show any abnormalities, and repetitive nerve stimulation at a rate of 3 Hz had no decremental response. Serum immunoglobulin G antibody to GQ1b was found to be positive. A clinical diagnosis of PCB was made, and the patient was treated with intravenous immunoglobulin (2 g/kg over 4 days). He was extubated successfully after a few days. Postextubation, his negative inspiratory force and peak expiratory flow were recorded as −40 mmHg and 250 ml/min, respectively.

He was transferred after 1 week to our rehabilitation center, where he still had similar cranial nerve and left upper arm neurological deficits. After 2 weeks of inpatient rehabilitation, his upper limb weakness had recovered, but he had persistent dysarthria and dysphagia despite daily speech therapy. He was taught to type on his handphone as an alternative communication mode. He still required a nasogastric tube. Regular atropine oral drops were prescribed for his oral secretions as he could not clear them due to impaired swallowing. Despite chest physiotherapy, he developed nosocomial pneumonia which resolved with intravenous antibiotics. His urinary catheter was removed successfully. On discharge from inpatient rehabilitation, his functional independence measure (FIM) score improved from 118 to 125. Unfortunately, he was unable to return to his premorbid occupation of an image consultant and a piano teacher due to his impairments.

At 3 months post illness, he remained severely dysarthric and dysphagic and was distressed at his lack of recovery. A fiberoptic endoscopic evaluation of swallowing (FEES) demonstrated silent aspiration across all diet and liquid consistencies. A repeat prognostic electrophysiological study was performed. It demonstrated a predominantly axonal nonlength-dependent polyneuropathy involving the bilateral facial nerves and tongue [Table 1], with needle electromyography showing ongoing denervation changes [Table 2]. Repetitive nerve stimulation over the nasalis and trapezius did not have abnormal decremental response. The electrophysiological features were consistent with a diagnosis of PCB.[1]
Table 1: Nerve conduction study at 3 months of illness

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Table 2: Electromyography at 3 months of illness

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He continued oromotor facilitation exercises and oral stimulation techniques with regular speech therapy reviews. He was also started on electrical stimulation therapy twice a week to the pharyngeal muscles (Biphasic, 6.0 mA, 60 Hz, 20 min each session).

Improvement in his dysarthria was only noted at 4 months post illness onset, along with a videofluoroscopic swallow study (VFSS) showing improved but still impaired pharyngeal dysphagia. He was taken off his nasogastric tube and started on a modified diet with cyclic ingestion techniques. He only achieved normal swallow function without the requirement of any compensatory maneuvers at 8 months post illness onset and was also able to return to his previous occupation successfully.


  Discussion Top


GBS classically presents with ascending symmetrical and flaccid paralysis. It most commonly manifests as an acute inflammatory demyelinating polyneuropathy although axonal subtypes such as acute motor axonal neuropathy and acute motor and sensory axonal neuropathy have been described. Other variants include acute pandysautonomia, Miller Fisher syndrome (ophthalmoplegia, ataxia, and areflexia), and the rare PCB subtype (oropharyngeal and cervicobrachial weakness).

Nearly half of all patients with GBS develop symptoms of bulbar palsy.[2] Although some may just experience mild motor weakness affecting oromotor manipulation, severe forms of GBS often have significant deficits in oropharyngeal function requiring enteral feeding. As dysphagia can last for up to 9 months in severe cases, percutaneous gastrostomy tubes are an option for patients to maintain adequate nutrition.[3] Intensive restorative and compensatory-oriented dysphagia strategies addressing bulbar weakness and airway protection deficits are often required to maximize outcomes.[4] To evaluate oropharyngeal impairment, objective assessment with FEES or VFSS is often recommended as the most effective modality.[5],[6] It can also be utilized to assess compensatory strategies, diet modification, secretion management, and airway protection.

Transcutaneous electrical stimulation is a noninvasive technique that augments weak muscles by stimulating purposeful muscle contraction, and there is emerging evidence that it also has a positive impact on muscles of swallowing.[5] Apart from stroke patients, electrical stimulation has been utilized in a patient with GBS [7] and patients with dysphagia with direct neurological damage from cancer or radiation.[8],[9] Electrical stimulation may not be effective in the acute stages of denervation in GBS. However, as the peripheral nerves recover, electrical stimulation to the pharyngeal muscles can be a useful therapeutic adjunct for neural activation to reduce muscle degeneration and motor dysfunction.[5],[10]

On the other hand, bulbar recovery in PCB is usually rapid and can occur with 2 weeks.[11] As such patients are expected to recover and transit to oral diet within days, simple oromotor exercises are often sufficient and enteral feeding, if required, can be accomplished via a short term nasogastric tube. Objective instrumental swallowing assessment may not be required in the event of rapid oropharyngeal improvement. However, some patients rarely require up to 2 months for recovery.[12] A recovery duration as long as 4 months in our patient has not been reported, to the best of our knowledge. Given the prolonged oropharyngeal impairment in our patient, dysphagia therapy had to be adapted from the aforementioned techniques employed in severe GBS, including instrumental swallowing assessment, daily oromotor stimulation and strengthening exercises, airway protection techniques, adjunctive electrical stimulation, and a staged and gradual advancement in diet consistencies. Percutaneous gastrostomy feeding should also be considered as an option if there is an anticipated need for prolonged enteral nutrition. As PCB patients often remain physically active, those dependent on tube feeding need frequent dietician reviews to meet the energy requirements of an active lifestyle. As our patient was at risk of aspiration for a considerable amount of time, regular chest physiotherapy and inspiratory muscle training were important during the acute and subacute rehabilitation phase, and pharmacological agents were provided to manage secretions.

No prognostic factors for the PCB subpopulation have also been described, although the following electrophysiological and clinical factors pointed toward a delayed recovery trajectory in our patient. The electrodiagnostic evidence of axonal injury of the oropharyngeal muscles indicated a poorer prognosis.[13] Ongoing denervation changes at 3-month follow-up also portended a delayed recovery. A complete PCB presentation is expected to be worse, given that incomplete variants with isolated oropharyngeal weakness or PCB/Miller Fisher overlap often have rapid oropharyngeal recovery within weeks.[11],[14] Our patient also was of an older age, required ventilatory support, had rapid disease progression, experienced urinary retention, and was on nasogastric tube feeding. These electrophysiological and clinical indicators have been shown to be associated with more severe clinical disease and poorer functional outcome in the general GBS population and are likely to be applicable to our patient as well.[15],[16],[17]

The majority of speech impairments in GBS result from the need of a tracheostomy, as approximately 20%–30% of patients with GBS experience respiratory impairment requiring prolonged mechanical ventilation. For these patients, the speech therapist should focus on secretion management, cuff deflation, and the introduction of a speaking valve with a view toward decannulation before recommencement of normal speech can happen.[4] Our patient did not require a tracheostomy, although his bulbar palsy resulted in impaired verbal communication. We found that augmentative and alternative communication (AAC) was effective and took precedence over task-specific and impairment-based dysarthria training even in the early phases of rehabilitation, as full spontaneous recovery is often expected in PCB. Our patient also had a poorer oropharyngeal recovery trajectory, and AAC allowed him to achieve community independence during his convalescent period.

This case illustrates the significant functional gains a patient with PCB can make despite a prolonged recovery trajectory. Early identification of poor prognostic factors and goal-specific rehabilitation in the inpatient and outpatient setting will assist patients with GBS and its variants in achieving independence and maximizing community reintegration. However, the validity of applying general GBS prognostic factors for rarer GBS variants needs further investigation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Wakerley BR, Yuki N. Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry 2014;85:339-44.  Back to cited text no. 1
    
2.
Winer JB. Guillain-Barré syndrome. BMJ 2008;337:a671.  Back to cited text no. 2
    
3.
Yoshida M, Ikeda J, Urikane Y, Kashiwada T, Kaseda Y, Kohriyama T. Prevalence of tracheotomy and percutaneous endoscopic gastrostomy in patients with Guillain-Barré syndrome. Dysphagia 2017;32:236-40.  Back to cited text no. 3
    
4.
Kazandjian M, Dikeman K. Guillain-Barré syndrome and disordered swallowing. Perspect Swal Dis (Dysph) 2012;21:115-20.  Back to cited text no. 4
    
5.
Clark H, Lazarus C, Arvedson J, Schooling T, Frymark T. Evidence-based systematic review: Effects of neuromuscular electrical stimulation on swallowing and neural activation. Am J Speech Lang Pathol 2009;18:361-75.  Back to cited text no. 5
    
6.
Dietrich-Burns K, Lewis WJ, Lesley DY, Solomon NP. Silent aspiration and recovery from dysphagia in a case of bickerstaff brainstem encephalitis. Mil Med 2013;178:e121-4.  Back to cited text no. 6
    
7.
Lee KL, Lim OK, Lee JK, Park KD. Treatment of dysphagia with pyridostigmine bromide in a patient with the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Ann Rehabil Med 2012;36:148-53.  Back to cited text no. 7
    
8.
Crary MA, Carnaby GD. Adoption into clinical practice of two therapies to manage swallowing disorders: Exercise-based swallowing rehabilitation and electrical stimulation. Curr Opin Otolaryngol Head Neck Surg 2014;22:172-80.  Back to cited text no. 8
    
9.
Tan C, Liu Y, Li W, Liu J, Chen L. Transcutaneous neuromuscular electrical stimulation can improve swallowing function in patients with dysphagia caused by non-stroke diseases: A meta-analysis. J Oral Rehabil 2013;40:472-80.  Back to cited text no. 9
    
10.
Al-Majed AA, Neumann CM, Brushart TM, Gordon T. Brief electrical stimulation promotes the speed and accuracy of motor axonal regeneration. J Neurosci 2000;20:2602-8.  Back to cited text no. 10
    
11.
Onodera M, Mori M, Koga M, Kamitsukasa I, Fukutake T, Hattori T, et al. Acute isolated bulbar palsy with anti-GT1a IgG antibody subsequent to campylobacter jejuni enteritis. J Neurol Sci 2002;205:83-4.  Back to cited text no. 11
    
12.
Arai M, Susuki K, Koga M. Axonal pharyngeal-cervical-brachial variant of Guillain-Barré syndrome without anti-GT1a IgG antibody. Muscle Nerve 2003;28:246-50.  Back to cited text no. 12
    
13.
González-Suárez I, Sanz-Gallego I, Rodríguez de Rivera FJ, Arpa J. Guillain-Barré syndrome: Natural history and prognostic factors: A retrospective review of 106 cases. BMC Neurol 2013;13:95.  Back to cited text no. 13
    
14.
Capasso M, Notturno F, Manzoli C, Yuki N, Uncini A. Reversible conduction failure in pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Muscle Nerve 2010;42:608-12.  Back to cited text no. 14
    
15.
Walgaard C, Lingsma HF, Ruts L, van Doorn PA, Steyerberg EW, Jacobs BC. Early recognition of poor prognosis in Guillain-Barre syndrome. Neurology 2011;76:968-75.  Back to cited text no. 15
    
16.
Ng YS, Lo YL, Lim PA. Characteristics and acute rehabilitation of Guillain-Barré syndrome in Singapore. Ann Acad Med Singapore 2004;33:314-9.  Back to cited text no. 16
    
17.
Yamagishi Y, Suzuki H, Sonoo M, Kuwabara S, Yokota T, Nomura K, et al. Markers for Guillain-Barré syndrome with poor prognosis: A multi-center study. J Peripher Nerv Syst 2017;22:433-9.  Back to cited text no. 17
    



 
 
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  [Table 1], [Table 2]



 

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